Ovary

Sex cord stromal tumors

Pure stromal tumors

Steroid cell tumor



Last author update: 3 April 2024
Last staff update: 3 April 2024

Copyright: 2002-2024, PathologyOutlines.com, Inc.

PubMed search: Ovarian steroid cell tumor

Fatemeh Ghazanfari Amlashi, M.D.
Tamara Kalir, M.D., Ph.D.
Page views in 2024 to date: 8,239
Cite this page: Amlashi FG, Kalir T. Steroid cell tumor. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/ovarytumorsteroidcelltumorNOS.html. Accessed June 16th, 2024.
Definition / general
  • Ovarian stromal tumor composed of steroid cells with malignant potential
Essential features
  • Mostly unilateral
  • Half of cases have androgenic manifestations
  • Microscopic features include diffuse sheets of polygonal to rounded tumor cells with moderate to abundant cytoplasm (Am J Surg Pathol 1987;11:835)
Terminology
  • Steroid cell tumor, not otherwise specified (NOS)
ICD coding
  • ICD-O
    • 8670/0 - steroid cell tumor, NOS
    • 8670/3 - steroid cell tumor, malignant
  • ICD-11: 2C73.Y & XH4L39 - other specified malignant neoplasms of the ovary & steroid cell tumor, malignant
Epidemiology
Sites
Pathophysiology
  • Tumors are presumed to be of ovarian stromal cell origin
Etiology
Clinical features
  • Androgenic (~50%), estrogenic changes including rare examples of isosexual pseudoprecocity (~10%), occasional progestogenic changes, Cushing syndrome or elevated cortisol levels (uncommon)
  • Rarely with hypercalcemia, erythrocytosis, ascites or acute heart failure
  • Reported in von Hippel-Lindau (both types I and II), with the most common presentation including abnormal uterine bleeding, amenorrhea and infertility (Int J Clin Exp Pathol 2022;15:332)
Diagnosis
  • Histologic examination
Laboratory
  • In patients with androgenic changes, Cushing syndrome or both: elevated urinary levels of 17-ketosteroids and 17-hydroxycorticosteroids, serum testosterone and androstenedione
  • In Cushing syndrome: elevated free cortisol in the blood or urine
Radiology description
Radiology images

Images hosted on other servers:
Enhancing adnexal mass Enhancing adnexal mass

Enhancing adnexal mass

Fat containing pelvic mass

Fat containing pelvic mass

Homogeneous isoechoic mass Homogeneous isoechoic mass

Homogeneous isoechoic mass

Solid adnexal mass

Solid adnexal mass

Prognostic factors
  • Clinically malignant in 25 - 43% of cases (Am J Surg Pathol 1987;11:835)
  • Patients with clinically malignant tumor were on average 16 years older than patients with benign tumors in one series (Am J Surg Pathol 1987;11:835)
  • No malignant steroid cell tumors have been reported in patients in their first 2 decades
  • Rare tumors have recurred up to 19 years postoperatively (Am J Surg Pathol 1987;11:835)
  • Extraovarian spread of tumor is present at the time of operation in a small minority of cases; 3 cases with Cushing disease with extensive intra-abdominal spread of tumor (Int J Gynecol Pathol 1987;6:40)
  • Features associated with malignancy (Am J Surg Pathol 1987;11:835)
    • 2+ mitoses per 10 high power fields (92% malignant)
    • Necrosis (86% malignant)
    • 7 cm or larger (78% malignant)
    • Intratumoral hemorrhage (77% malignant)
    • Grade 2 or 3 nuclear atypia (64% malignant)
  • Aggressive behavior with 4 or more malignant features (Am J Surg Pathol 2023;47:1398)
Case reports
Treatment
Clinical images

Images hosted on other servers:
Hirsutism

Hirsutism

Male pattern baldness

Male pattern baldness

Gross description
  • Wide size range (mean size: 8.4 cm) (Am J Surg Pathol 1987;11:835)
  • Unilateral (95%)
  • Solid, well circumscribed, occasionally lobulated
  • Sections from yellow-orange (lipid rich) to red-brown (lipid poor) to dark brown-black (abundant lipochrome pigment)
  • Occasionally with necrosis, hemorrhage and cystic degeneration
Gross images

Images hosted on other servers:
Yellow cut surface

Yellow cut surface

Metastatic steroid cell tumor

Metastatic steroid cell tumor

Lobulated cut surface

Lobulated cut surface

Nodular solid tumor

Nodular solid tumor

Tan-brown, well circumscribed tumor with hemorrhage

Tan-brown, well circumscribed tumor with hemorrhage

Frozen section description
  • Diffuse or nested arrangement of tumor cells
  • Cells with abundant eosinophilic or clear, foamy cytoplasm
Frozen section images

Contributed by Fatemeh Ghazanfari Amlashi, M.D. and Tamara Kalir, M.D., Ph.D.
Monotonous sheets of cells

Monotonous sheets of cells

Abundant cytoplasm

Abundant cytoplasm

Microscopic (histologic) description
  • Architectural pattern: diffuse (most common) but occasionally in nests, cords, pseudoglandular and follicle-like arrangements (Am J Surg Pathol 1987;11:835)
  • Stroma: usually sparse (85%) but may be fibrotic or hyalinized, rarely edematous or myxoid, exceptionally with calcification and psammoma body formation
  • Polygonal to rounded tumor cells with distinct cell borders, central nuclei and moderate to abundant cytoplasm
  • Tumor cell cytoplasm: eosinophilic and granular (lipid poor) to vacuolated and spongy (lipid rich); tumors with a mixture of 2 cell types are also present
  • More lipid rich cytoplasm in this tumor compared to other subtypes of steroid cell tumor
  • Lipochrome pigment present (40%)
  • Rarely, signet ring appearance
  • Adipocytic metaplasia and hyaline globule (unusual) (Am J Surg Pathol 2023;47:1398)
  • Usually absent / slight nuclear atypia along with low mitotic activity (< 2 mitotic figures/10 high power fields); those with grades 1 - 3 nuclear atypia associated with increased mitotic activity (up to 15 mitotic figures/10 high power fields)
  • Necrosis and hemorrhage are occasionally present, particularly in tumors with significant cytologic atypia
  • Metastatic tumor is similar to the primary tumor in some cases but more poorly differentiated in others
  • Stromal hyperthecosis may be seen in the adjacent ovarian stroma and contralateral ovary, particularly in small tumors
Microscopic (histologic) images

Contributed by Fatemeh Ghazanfari Amlashi, M.D., Tamara Kalir, M.D., Ph.D. and AFIP
Well defined tumor

Well defined tumor

Sparse stroma

Sparse stroma

Diffuse sheets

Diffuse sheets

Moderate to abundant cytoplasm

Moderate to abundant cytoplasm

Round to polygonal cells

Round to polygonal cells

Distinct cell borders

Distinct cell borders


Spongy and eosinophilic cytoplasm

Spongy and eosinophilic cytoplasm

2 atypical mitotic figures

2 atypical mitotic figures

Inhibin

Inhibin

Calretinin

Calretinin

MelanA

MelanA

Abundant lipid (oil red O stain)

Abundant lipid (oil red O stain)

Virtual slides

Images hosted on other servers:
Lipid rich tumor cells

Lipid rich tumor cells

Cytology description
  • Large polygonal to round cells
  • Arranged in sheets and attached with vascular stromal tissue fragments (J Cytol 2015;32:284)
  • Cells with small central round nuclei with conspicuous nucleoli and abundant granular to pale foamy cytoplasm
  • Ascites in malignant steroid cell tumor: isolated tumor cells or clusters with slight overlapping and cannibalism formation (Acta Cytol 2017;61:165)
Cytology images

Images hosted on other servers:
Large polygonal cells

Large polygonal cells

Molecular / cytogenetics description
Videos

Steroid cell tumor

Sample pathology report
  • Right ovary and fallopian tube, salpingo-oophorectomy:
    • Ovarian steroid cell tumor, not otherwise specified (6 cm size) (see comment)
    • Fallopian tube with no significant pathologic change
    • Comment: Sections show a well circumscribed neoplasm composed of abundant clear cytoplasm. There is mild nuclear atypia. No mitosis or necrosis identified. Immunostains show the tumor cells are positive for inhibin, calretinin and negative for CD10, supporting the diagnosis.

  • Left ovary and fallopian tube, salpingo-oophorectomy:
    • Ovarian steroid cell tumor, not otherwise specified (12 cm size) (see comment)
    • Fallopian tube with no significant pathologic change
    • Comment: Sections show a well circumscribed neoplasm composed of sheets of round cells with abundant eosinophilic granular cytoplasm. Intratumoral hemorrhage and focal necrosis is present. Moderate nuclear atypia and mitoses (4/10 high power fields) are seen. The ovarian capsule is intact and uninvolved by the tumor cells. Immunostains show the tumor cells are positive for calretinin and inhibin, supporting the diagnosis.
Differential diagnosis
Board review style question #1

Which of the following statements is correct regarding steroid cell tumor, not otherwise specified (NOS) of the ovary?

  1. Inhibin+, calretinin+, MelanA+, PAX8-
  2. Less than 1% risk of malignancy
  3. Mostly bilateral
  4. Various architectural patterns
Board review style answer #1
A. Inhibin+, calretinin+, MelanA+, PAX8-. Steroid cell tumor, NOS shows positive inhibin, calretinin and MelanA and negative PAX8. Note that MelanA (A103 clone) can be expressed in steroid cell tumor and the presence of it should not necessarily prompt the diagnosis of melanoma. Answer B is incorrect because steroid cell tumor has a 30% risk of malignancy. Answer C is incorrect because steroid cell tumors are mostly unilateral. Answer D is incorrect because having various architectural patterns is a feature of ovarian clear cell carcinoma.

Comment Here

Reference: Steroid cell tumor
Board review style question #2
A 40 year old woman presented with 5 cm, solid, well defined adnexal mass. Which of the following features is in favor of steroid cell tumor, NOS?

  1. Acellular eosinophilic material and nuclear clustering
  2. Location in hilum
  3. Reinke crystal
  4. Sheets of tumor cells with distinct cell borders and clear to foamy cytoplasm
Board review style answer #2
D. Sheets of tumor cells with distinct cell borders and clear to foamy cytoplasm. Steroid cell tumor, NOS is mostly seen as sheets of tumor cells with distinct cell borders and can have clear to foamy cytoplasm in lipid rich tumor. Answers A, B and C are incorrect because these are features of Leydig cell tumor.

Comment Here

Reference: Steroid cell tumor
Back to top
Image 01 Image 02